Download Skin Necrosis as a Predictive Factor for Neoplasia in

Document downloaded from http://www.elsevier.es, day 11/06/2017. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.
CASE AND RESEARCH LETTERS
Skin Necrosis as a Predictive Factor
for Neoplasia in Dermatomyositis
Necrosis cutánea como factor predictivo
de neoplasia en la dermatomiositis
To the Editor:
Dermatomyositis (DM) is a rare myopathic disorder
characterized by symmetric proximal muscle weakness
and characteristic skin lesions; it is associated with an
underlying neoplasm in up to 30% of cases.1-7 Several studies
have recently been aimed at identifying demographic,
clinical, and laboratory data that could predict the
individual risk of malignant tumors in patients with DM.
Several authors agree in considering the appearance
of necrotic skin lesions as a potential risk factor for
paraneoplastic DM.
We report the case of a 58-year-old man who came
to our department with a 2-month history of symmetric
muscle weakness associated with multiple erythematouspurpuric papules over the interphalangeal joints of both
hands (Gottron papules) and asymptomatic edematous
violaceous plaques (heliotrope rash) (Figure 1A).
Other skin findings were periungual telangiectasia and
hypertrophy of the nail cuticle Figure 1B). The most
relevant clinical finding consisted of extensive areas of
slightly painful skin necrosis in a symmetrical distribution
over the upper third of the thorax and back (Figure 1C).
Histology showed an atrophic epidermis, mild interface
dermatitis with vacuolar degeneration of the basement
membrane, and a superficial perivascular lymphocytic
inflammatory infiltrate accompanied by interstitial
deposits of mucin. Laboratory findings, with elevated
levels of muscle enzymes (creatine phosphokinase, 5280
U/L) and aldolase (47.5 U/mg) were compatible with a
diagnosis of DM.
455
The presence of areas of necrosis and the age of the
patient were suggestive of a paraneoplastic origin of
the DM. Computed tomography revealed a gastric tumor
with liver metastases (Figure 2). The patient received
chemotherapy, including 5-fluorouracil, anthracyclines,
and cisplatin; the chemotherapy reduced the tumor mass
and the skin lesions resolved.
The definition of predictive factors for malignancy in adult
DM will make it possible to more accurately select those
patients in whom an exhaustive search for a tumor must
be performed. The epidemiologic, clinical, and laboratory
findings that are currently considered as potential markers
for paraneoplastic DM are shown in the Table.1-7 Several
studies show that skin necrosis is an important independent
predictive factor for an underlying tumor in patients with
DM.1-4 In the study by Sparsa et al,2 both of the 2 patients who
presented skin necrosis had associated neoplasia. A recent
analysis based on the study of 26 patients with DM found a
positive predictive value of 71.4% for the association of skin
necrosis and cancer.4 The clinical spectrum of skin necrosis
includes epidermal necrosis, digital necrosis (periungual and
of the digital pulp), and mucosal necrosis.4
Our case and the previously published series1-4 suggest
that this clinical parameter, which can be easily identified
by the dermatologist, is probably the most important
indication for an exhaustive and detailed investigation
for underlying cancer in DM. Although ovarian cancer is
currently considered to be the malignant tumor most
frequently associated with paraneoplastic DM, other tumors
that are reported in the literature and need to be ruled out
are lung cancer, pancreatic cancer, and gastrointestinal
cancer. Testicular cancer is the most prevalent cancer in
young patients.1-7 Finally, nasopharyngeal cancer should be
ruled out in Asian patients.5
In conclusion, the development of necrotic lesions in the
context of dermatomyositis is a rare occurrence. However,
regardless of the age of the patient at onset of the disease,
these lesions require an exhaustive study to rule out an
underlying tumor.
Figure 1 A, Asymptomatic erythematous-violaceous plaques located symmetrically around the eyes. B, Gottron papules and
hypertrophic nail cuticles with areas of necrosis. C, Extensive skin necrosis with a symmetrical distribution on the trunk.
Document downloaded from http://www.elsevier.es, day 11/06/2017. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.
456
CASE AND RESEARCH LETTERS
Conlicts of Interest
The authors declare that they have no conflicts of
interest.
References
Figure 2 Computed tomography image. Areas of increased
uptake compatible with gastric cancer with liver metastases (red
arrows).
Table 1 Predictive Factors for Malignancy in
Dermatomyositis and Polymyositis
Personal history of a previous tumor
Sudden onset of skin/muscle symptoms
Skin necrosis*
Constitutional symptoms
Laboratory parameters:
Elevated levels of CPK (>1000 U/L)
Low C4 counts (<40 mg/dL)
Elevated p155/140 antibody titer
*Includes epidermal necrosis, digital necrosis (periungual
and of the digital pulp), and mucosal necrosis.
Abbreviation: CPK, creatine phosphokinase.
Dermoscopic indings in solitary
reticulohistiocytosis
Hallazgos en dermatoscopia del
reticulohistiocitoma cutáneo solitario
To the Editor:
Solitary
cutaneous
reticulohistiocytosis,
initially
described by Zak in 1959,1 is a variant of multicentric
reticulohistiocytosis that is limited to the skin. It is a
rare condition that is characterized by rapid growth of a
single brownish-yellow or reddish lesion, which is usually
1. Fardet L, Dupuy A, Gain M, Kettaneh A, Chérin P, Bachelez H, et
al. Factors associated with underlying malignancy in
retrospective cohort of 121 patients with dermatomyositis.
Medicine. 2009;88:91-7.
2. Sparsa A, Liozon E, Herrmann F, Ly K, Lebrun V, Soria P, et al.
Routine vs extensive malignancy search for adult dermatomyositis
and polymyositis: a study of 40 patients. Arch Dermatol.
2002;138:885-90.
3. Callen JP. When and how should the patient with dermatomyositis
or amyopathic dermatomyositis be assessed for possible cancer?.
Arch Dermatol. 2002;138:969-71.
4. Mahé E, Descamps V, Burnouf M, Crickx B. A helpful clinical sign
predictive of cancer in adult dermatomyositis: cutaneous
necrosis. Arch Dermatol. 2003;139:539.
5. Chen YJ, Wu CY, Shen JL. Predicting factors of malignancy in
dermatomyositis and polymyositis: a case-control study. Br J
Dermatol. 2001;144:825-31.
6. Bielsa I. Nuevos anticuerpos en la dermatomiositis. Actas
Dermosiiliogr. 2009;100:182-9.
7. Chinoy H, Fertig N, Oddis CV, Ollier WE, Cooper RG. The
diagnostic utility of myositis autoantibody testing for predicting
the risk of cancer-associated myositis. Ann Rheum Dis. 2007;
66:1345-9.
A. Martorell-Calatayud,* C. Serra Guillén,
C. Ciudad-Blanco, and O. Sanmartín
Servicio de Dermatología, Instituto Valenciano
de Oncología, Valencia, Spain
*Corresponding author.
E-mail address: [email protected]
(A. Martorell-Calatayud).
asymptomatic. It typically presents on the trunk or the
limbs, and rarely on the face.2 Histology is characterized
by a dermal infiltrate of histiocytes with an eosinophilic
cytoplasm with a ground-glass appearance and prominent
nucleoli.2 An inflammatory infiltrate composed mainly
of lymphocytes is also observed. Hyperkeratosis and,
occasionally, parakeratosis may be observed. Immune
staining is positive for lysozyme, CD68, and CD163 and
negative for CD3, CD20, CD30, human melanoma black-45,
and keratins. The condition is not associated with other
diseases and recurrence after excision is very rare.
We report the case of a 51-year-old man with no relevant
personal history other than hypercholesterolemia, which
was being treated with simvastatin. He reported the
sudden appearance of an asymptomatic papular lesion